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Maternal connective tissue disorders 

Maternal connective tissue disorders
Chapter:
Maternal connective tissue disorders
Author(s):

Harini Narayan

DOI:
10.1093/med/9780199673643.003.0014
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date: 26 November 2020

Marfan’s syndrome and Ehlers-Danlos syndrome (EDS), inherited as mainly autosomal dominant conditions, can have profound effects on pregnancy and may have life-threatening complications including aortic dissection. Prognosis depends on the various types (EDS) and manifestations, especially cardiovascular involvement. This chapter describes the epidemiology, genetics, clinical features, diagnosis of both conditions, their impact on pregnancy and vice versa. Multidisciplinary management from pre-pregnancy to post-delivery, encompassing primary, secondary, and tertiary care, is presented in a format suitable for use in busy clinical settings. Pre-conceptual assessment and counselling and continued assessments and vigilance to detect complications and their immediate management are described. Individualized care pathways provide guidance regarding appropriate referral to tertiary centres for integrated care. Essential documentation and communication of key information for intrapartum and postnatal care including avoidance of unnecessary interventions, are highlighted. Patient information is provided to enhance her awareness of warning symptoms of life-threatening complications.

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